Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-seven cases of primary peripheral T-cell lymphomas of the intestine (PTLI) were investigated. Seven patients had histories of malabsorption. The most frequent symptoms at presentation were weight loss, abdominal pain, and acute abdomen. The jejunum was the most common site of lymphoma and multifocal disease was found in 72% of the cases. Twenty-two patients (92%) presented with localized disease confined to the intestine and abdominal lymph nodes, only two patients had generalized disease. According to the pattern of lymphoma infiltration and the morphology of the uninvolved small intestinal mucosa, 21 cases were separated histologically into three categories; 1) enteropathy-associated T-cell lymphoma (EATCL, n = 9) showing predominant intramucosal lymphoma spread and villous atrophy of uninvolved mucosa with high density of intraepithelial lymphocytes (IEL), 2) EATCL-like lymphoma without enteropathy (EATCL-LLWE, n = 5) but with an infiltration pattern similar to EATCL, and 3) T-cell lymphoma without features of EATCL (Non-EATCL, n = 7). Distinctive features of EATCL were the high incidence of malabsorption states, multifocal intestinal disease in all cases, and the high frequency of intestinal recurrences. On frozen sections four of eight PTLI showed the phenotype CD3+ CD4- CD8- HML-1+, which is also expressed on a small subset of normal IEL. The morphologic and immunomorphologic findings suggest that the majority of PTLI is derived from mucosal T lymphocytes. This derivation may be responsible for certain biologic features, such as the preferential spread to and relapse of PTLI at small intestinal sites.
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PMID:Peripheral T-cell lymphomas of the intestine. 146

Enteropathy-associated T-cell lymphoma commonly presents with malabsorption, and debate continues as to whether adult-onset coeliac disease (CD) is itself a form of low-grade lymphoma. A 59-year-old man with adult-onset CD required resection of a segment of oedematous jejunum. Histological examination of this tissue revealed an intense intraepithelial lymphocytosis. Immunophenotypic (CD3-, CD4-, CD8-, CD34-, and CD45 RO-) and cytogenetic (deletion of the Y chromosome and chromosome 9) abnormalities were found, together with monoclonal T-cell-receptor gene rearrangements. Some patients with adult-onset CD may have low-grade lymphoma from the outset of their illness.
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PMID:Is adult-onset coeliac disease due to a low-grade lymphoma of intraepithelial T lymphocytes? 167 39

A prospective study of 60 consecutively admitted patients with HIV infection was performed to document the prevalence, etiology and manifestations of low serum vitamin B-12 in such patients. Low serum B-12 levels were found in 10 patients (16.7%). In 6, vitamin B-12 absorption was impaired and hog intrinsic factor addition did not improve it. Patients with low vitamin B-12 levels showed lower hemoglobin, leukocytes, lymphocytes, CD4 lymphocytes and CD4/CD8 lymphocyte ratio than HIV patients with physiological serum vitamin B-12 levels. However, bone marrow megaloblastosis was found in only 3 low vitamin B-12 patients and the deoxyuridine suppression test was pathological in only 1 case. In 7 patients, parenteral treatment was begun with variable response despite serum vitamin B-12 correction. In conclusion, low serum vitamin B-12 is often found in HIV-infected patients and it could be related to malabsorption, but clear megaloblastic abnormalities and treatment response could not be demonstrated. A decreased concentration of the serum binders due to disturbances in the leukocytes and related immunocompetent cell may play an additional role.
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PMID:Vitamin B-12 abnormalities in HIV-infected patients. 186 15

Newborns suffering from short bowel syndrome (SBS) after massive intestinal resection have numerous infectious complications, which may be due to immunoincompetence from the loss of gut-associated lymphoid tissue (GALT). This study examines the ontogeny of GALT in the rat with SBS. A total of 36 3-week-old rats were divided into two groups: I, sham operated (C, n = 16); and II (SBS, n = 20), with a 50% resection of small intestine with jejunoileostomy. At 4, 5, 6, and 10 weeks of age the animals were sacrificed and the GALT was assessed by video analysis and immunoperoxidase monoclonal antibodies, OX8 (CD8), W-3/25 (CD4), and MARA-2 (IgA). The data were expressed as positive staining lymphocytes per 10(4) microns2 (mean +/- SD). table; see text In the sham group there was an increase in all lymphocyte subsets over time. In the SBS group there was a rapid fall in OX8 and W-3/25 T-lymphocytes by 10 weeks, with no increase in IgA plasma cells at 6 and 10 weeks. This study demonstrates that in the massive bowel resection in the suckling rat decreases the T- and B-lymphocyte populations in the GALT. This lack of development may underlie the associated infectious complications and malabsorption in SBS.
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PMID:The ontogeny of the gut-associated lymphoid tissue in short bowel syndrome. 233 23

We describe a patient with idiopathic hypereosinophilic syndrome, without initial gastrointestinal symptoms, and their transition to eosinophilic gastroenteritis. This patient, a 65-year-old man, presented with fever, constitutional symptoms, peripheral and bone marrow eosinophilia 20 years ago. During the course of the disease, diarrhoea and malabsorption became prominent, whereas bone marrow eosinophilia regressed completely and blood eosinophilia regressed partially. Biopsies showed a severe eosinophilic gastroenteritis of the mucosal type involving the stomach, small bowel and colon. During the final years of the patient's disease, mucosal eosinophilia became less intense and a mucosal infiltration with T-cells dominated. At autopsy, immunopathological studies of small intestines and colon specimens showed a clonal expansion of morphologically normal T-cells in the intestinal mucosa, which expressed the abnormal phenotype CD2+CD3+CD4-CD5-CD8-. Flow cytometry examination of peripheral blood revealed a corresponding abnormal population of CD3+CD4-CD8- T-cells, indicating a systemic spread of the process. The patient eventually died of non-obstructive small bowel infarction with peritonitis 20 years after the onset of the first symptoms. We postulate that the destructive eosinophilic/lymphocytic inflammation is caused by a clonal proliferation of T-lymphocytes with probable secretion of Type 2 T(helper) cell cytokines and consecutive stimulation of eosinophils.
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PMID:Long-term outcome of idiopathic hypereosinophilic syndrome--transition to eosinophilic gastroenteritis and clonal expansion of T-cells. 872 26

Two adult cases of extranodal malignant lymphoma diffusely involving the intestinal wall are reported. Lymphoblastic lymphoma in case 1 (68-year-old male) and small lymphocytic lymphoma in case 2 (48-year-old male) were initially manifested as protein-losing enteropathy or malabsorption syndrome. In case 1, the patient died of massive ascites and intestinal bleeding 10 months after presentation. Autopsy revealed diffuse and extensive lymphomatous involvement of the small and large intestine, peritoneum and liver. The patient in case 2, showing low-grade diffuse lymphomatous invasion throughout the small bowel mucosa without involving other organs, is currently being followed up. Surface marker studies revealed previously undescribed phenotypes, such as CD4/CD8 double-positive blastoid T cells in case 1, and CD8-positive suppressor/killer T cells in case 2. T-cell receptor alpha/beta was expressed on the lymphoma cells in both cases. Epstein-Barr virus infection was not evident. The gut-associated nature of the T-lymphoma cells, possibly of extrathymic origin, is discussed.
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PMID:T-cell lymphomas diffusely involving the intestine: report of two rare cases. 876 87

The polyglandular autoimmune syndromes (PGA) are well known and are distinguished into type I, type II and type III. PGAI, also called APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy), is an autosomal recessive disorder, appearing in childhood and typically characterized by hypoparathyroidism (unusual in PGAII and PGAIII) and adrenal insufficiency. In APECED, autoimmune destruction of the pancreatic beta cells with development of insulin-dependent type 1 diabetes is possible, but less frequent than in the other PGAs, especially PGAII. The pathogenesis of this unique autoimmune disease is unknown. No HLA association seems to exist and genetic studies have assigned the autosomal APECED locus to chromosome 21. The case of a 28-years-old female suggesting the diagnosis of APECED, is presented, characterized by psycho-somatic abnormal development, teeth alterations, post-puberal gonadal failure with dystrophic hypoplasia of external genitalia, previous vaginal candidiasis, a slowly developing juvenile brittle diabetes. Intestinal malabsorption induced by Giardia lamblia occurred (probably resulting, like candidiasis, from immunological anergy). A strong familiarity linked to female sex was noticed (the mother, a sister, the little nice and some maternal female cousins being affected) while the father and a brother were healthy. Diabetes seems to be characterized by early onset and severe complications. In this patient no organo-specific antibodies were detected and the only immunologic disorder was a small decrease of CD3 and CD4/CD8 ratio, both CD4 and CD8 being at the lower normal range. This patient (and her female maternal relatives) needs a long-term follow-up in order to evaluate the function of endocrine glands and to initiate early treatment for hormonal deficits, as well as to detect the non-endocrine components of disease.
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PMID:[A rare case of juvenile diabetes mellitus associated with APECED (autoimmune poly-endocrinopathy, candidiasis and ectodermal dystrophy) with strong X-linked familial inheritance]. 930 48

In our study, 7 bioptical specimens of intestinal T-cell lymphoma are described. This tumour occurs in the small intestine of adults who may have a history of malabsorption. The patients present clinically with intestinal perforation, enterorrhagia, or ileus. The gross appearance varies: the tumour may take the form of multiple ulcers or of a huge exulcerated lesion. The cytomorphological features are variable. Bizarre multinucleated cells may be sometimes present. The reactive cellular background is usually dominated by histiocytes or eosinophilic granulocytes. Immunohistochemically, the tumour cells stain in reactions with antibodies to pan T-cell markers, occasionally to CD8 and CD30. The positivity of tumour cells for markers of intraepithelial T-cells and for cytotoxic molecules has been demonstrated previously.
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PMID:[Intestinal T-cell lymphoma]. 1097 43

We studied the cellular immune response against malabsorption syndrome (MAS) in two broiler chicken lines, A and B. We determined the number of pan T-lymphocytes (CD3), helper T-lymphocytes (CD4), cytotoxic T-lymphocytes (CD8) and macrophages/monocytes in the small intestine in the first 2 weeks after oral inoculation of two MAS homogenates, MAS80 and MAS97-1. The immune cells were detected on cryostat tissue by immunohistochemistry and counted by villus area. In trial 1, we compared the two broiler lines for weight gain depression, intestinal lesion and number of CD3, CD4, CD8 cells and macrophages/monocytes after MAS80 inoculation. Although there was no significant difference in weight gain depression between the two broiler lines, line B had significantly higher numbers of CD8+ T-cells per villus area than had line A. To confirm part of the results of trial 1, trial 2 was done in which we compared different homogenates in broiler line B. Broiler line B was orally inoculated with either MAS97-1, intestinal homogenate obtained from healthy chickens (healthy homogenate), or phosphate buffered saline (PBS). In this trial, the MAS97-1 homogenate also induced weight gain depression and intestinal lesions, whereas the "healthy homogenate" and PBS did not induce weight gain depression or intestinal lesions. The broilers inoculated with MAS97-1 homogenate had significantly more CD8+ T-cells per villus area than had broilers inoculated with "healthy homogenate" or PBS. Increased CD8+ T-cells per villus area in the affected small intestines of broilers suggests an increase of cytotoxic T-cell activity.
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PMID:Cellular immune response in the small intestine of two broiler chicken lines orally inoculated with malabsorption syndrome homogenates. 1186 67

A primary small intestinal natural killer (NK) cell lymphoma with pathologic features of enteropathy but lack of association with celiac disease is reported. A 37-year-old man presented with tarry stool, coffee-ground vomitus, and mild fever. He did not have chronic diarrhea or malabsorption. Segmental resection of the duodenum and jejunum showed multicentric transmural infiltration by medium-sized lymphoma cells expressing CD3, CD8, cytotoxic granules, and Epstein-Barr virus by in situ hybridization. The nontumorous mucosa away from the main tumor revealed enteropathy with villous blunting and increased intraepithelial lymphocytes sharing the same immunophenotype as the lymphoma cells. Both lymphoma and nontumorous areas were germline for T-cell receptor-gamma and immunoglobulin heavy chain gene rearrangement. Serologic test by ELISA was negative for anti-transglutaminase. The patient died of repeated gastrointestinal bleeding and sepsis at 2 months. Differential diagnosis of this unique nasal-type NK-cell lymphoma with enteropathy-associated T-cell lymphoma is discussed.
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PMID:Natural killer cell lymphoma of small intestine with features of enteropathy but lack of association with celiac disease. 1513 43


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